期刊
MYASTHENIA GRAVIS AND RELATED DISORDERS: 11TH INTERNATIONAL CONFERENCE
卷 1132, 期 -, 页码 84-92出版社
BLACKWELL PUBLISHING
DOI: 10.1196/annals.1405.020
关键词
acetylcholine receptor; muscle-specific kinase; myasthenia gravis; complement; immunoglobulin G; animal model; IgG subclass
资金
- Medical Research Council [G117/490] Funding Source: Medline
- MRC [G117/490] Funding Source: UKRI
Antibodies to muscle-specific kinase (MuSK) are found in a variable proportion of patients with myasthenia without typical acetylcholine receptor (AChR) antibodies, but their characteristics and pathogenic mechanisms are not fully understood. We discuss the incidence and pathogenicity of MUSK antibodies and how clinical studies, animal models, and cultured cell lines can be used to elucidate their pathogenic mechanisms. Patients without either AChR or MUSK antibodies (seronegative myasthenia) appear to present another disease subtype that is highly similar to that of typical myasthenia gravis. We demonstrate a new method that detects AChR antibodies in these patients and show that these low-affinity AChR antibodies are predominantly IgG1 and can activate complement Cab deposition. Similarly MUSK antibodies, although mainly IgG4, are partially IgG1 and can activate Cab deposition. Overall, these results suggest that complement-activation may be an important pathogenic mechanism even in patients without conventional AChR antibodies.
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